Genetic Determinants of Kawasaki Disease

Genetic Determinants of Kawasaki Disease for Susceptibility and Outcome

Sponsors

Lead Sponsor: Imperial College London

Collaborator: Guy's and St Thomas' NHS Foundation Trust
University Hospitals Bristol NHS Foundation Trust
Great Ormond Street Hospital for Children NHS Foundation Trust
UK Kawasaki Support Group
Imperial College Healthcare NHS Trust
University Hospitals of North Midlands NHS Trust
Burton Hospitals NHS Foundation Trust
York Teaching Hospitals NHS Foundation Trust
The Shrewsbury and Telford Hospital NHS Trust
East Suffolk and North Essex NHS Foundation Trust
New Cross Hospital
Darlington Memorial Hospital
Torbay Hospital
Peterborough City Hospital
University Hospitals Coventry and Warwickshire NHS Trust
University Hospital of North Tees
Cambridge University Hospitals NHS Foundation Trust
Royal Cornwall Hospitals Trust
Birmingham Children's Hospital
Birmingham Heartlands Hospital
Wye Valley NHS Trust
South Tees Hospitals NHS Foundation Trust
Leeds General Infirmary
Bradford Royal Infirmary
University Hospitals of Morecambe Bay NHS Trust
Calderdale and Huddersfield NHS Foundation Trust
St. Richard's Hospital
Pinderfields General Hospital
Worthing Hospital
Airedale General Hospital
Sheffield Children's NHS Foundation Trust
Northwick Park Hospital
West Middlesex Hospital
Royal Albert Edward Infirmary
Macclesfield District General Hospital
Royal Oldham Hospital
Stepping Hill Hospital
North Manchester General Hospital
Royal Bolton Hospital NHS Foundation Trust
Kingston Hospital NHS Trust
Tameside Hospital NHS Foundation Trust
East Surrey Hospital
St. George's Hospital, London
Royal Alexandra Children's Hospital

Source Imperial College London
Brief Summary

Kawasaki disease (KD) is an acute self-limited vasculitis of infancy and early childhood. Most patients recover without sequelae although the inflammatory process causes permanent damage to the coronary arteries in 20-25% of untreated children. An infectious aetiology is suspected, but the causative agent has not been identified. The investigators aim to identify the genes underlying both susceptibility to Kawasaki disease, and the development of coronary artery aneurysms.

Detailed Description

The problem to be addressed;

Kawasaki disease is now the most common cause of acquired heart disease in children in Japan and North America. Kawasaki disease arises when genetically predisposed children encounter an as yet unidentified infectious agent which may cause only mild illness or no illness at all in children without the genetic predisposition. Other children may suffer permanent damage to the coronary arteries. Identification of the genes involved will help to improve understanding of the disease, and the development of better treatments.

Objective

The investigators aim to identify the genes underlying both susceptibility to Kawasaki disease, and the development of coronary artery aneurysms.

Design

The study will recruit nuclear families (affected child and their biological parents) through participating NHS hospitals or through the records of the UK Kawasaki Support Group.

Study size

400 affected children and both biological parents (i.e. 1200 participants)

Procedures

Informed consent using age appropriate patient/parent/guardian information sheets will be taken from parents (or from children aged 16 and over), assent will be taken from the child under the age of 16 (if appropriate). Children recruited during the acute illness; routine clinical and laboratory data and research samples (blood, urine, throat swab). Children recruited retrospectively; study questionnaire (completed by parents), saliva samples.Saliva samples from parents

Overall Status Recruiting
Start Date February 25, 2013
Completion Date December 30, 2022
Primary Completion Date December 30, 2022
Study Type Observational
Primary Outcome
Measure Time Frame
Susceptibility of coronary artery aneurysms for Kawasaki patients end date; 30 December 2022
Secondary Outcome
Measure Time Frame
Disease severity for Kawasaki patients end date; 30 December 2022
Enrollment 1299
Condition
Eligibility

Sampling Method: Probability Sample

Criteria:

Inclusion Criteria:

- Affected children will be recruited if the treating clinician has made a diagnosis of possible Kawasaki disease (even if they do not fulfil the criteria below for Kawasaki disease).

The current standard diagnostic criteria for KD (Circulation 2001 103 335-336 doi: 10.1161/01.CIR 103.2.335) are:

The presence of fever for at least five days plus four of the following criteria:

1. Changes in the peripheral extremities Acute: erythema and oedema of hands and feet Convalescent: membranous desquamation of fingertips

2. Polymorphous exanthema

3. Bilateral painless bulbar conjunctival injection without exudate

4. Changes in lips and oral cavity: erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae

5. Cervical lymphadenopathy (>1.5cm diameter), usually unilateral Patients meeting not all of these criteria may meet the criteria for atypical Kawasaki disease, ie. if they have fever and two or three of the above criteria and elevation of CRP or echocardiographic evidence of coronary artery dilatation.

Parents of affected child must be biological parents.

Exclusion Criteria:

- children who do not have a diagnosis of possible Kawasaki disease

Gender: All

Minimum Age: N/A

Maximum Age: N/A

Healthy Volunteers: No

Overall Official
Last Name Role Affiliation
Professor M Levin Study Chair Imperial College London
Overall Contact

Last Name: Jethro Herberg

Email: [email protected]

Location
Facility: Status: Contact: Contact Backup: Investigator: Imperial College London Jethro Herberg [email protected] Michael Levin Principal Investigator Herberg Jethro Principal Investigator
Location Countries

United Kingdom

Verification Date

August 2019

Responsible Party

Type: Sponsor

Keywords
Has Expanded Access No
Condition Browse
Arm Group

Label: Kawasaki disease

Description: Kawasaki disease affected childrens

Patient Data No
Study Design Info

Observational Model: Cohort

Time Perspective: Other

Source: ClinicalTrials.gov